Sprengel deformity


Sprengel deformity

Description, Causes and Risk Factors:

Sprengel deformity is a complex anomaly that is associated with malposition and dysplasia of the scapula (a large triangular flattened bone lying over the ribs, posteriorly on either side, articulating laterally with the clavicle at the acromioclavicular joint and the humerus at the glenohumeral joint. It forms a functional articulation with the chest wall, the scapulothoracic articulation). This condition also involves regional muscle hypoplasia or atrophy, which causes disfigurement and limitation of shoulder movement.

The etiology of Sprengel's deformity is related to limb bud formation. The scapula generally descends to its thoracic location about the eighth postgestational week, having formed from paraxial mesoderm at about the level of the fourth or fifth cervical vertebra. The usual postmigrational location of the scapula is between the levels of the second and eighth posterior ribs. Since Sprengel's deformity originates in the paracervical mesoderm, it is not surprising that Klippel-Feil syndrome and congenital scoliosis are frequent concomitants. Other rib and spinal anomalies, including syringomyelia, may be present. Diastematomyelia has been noted in about 20% of patients with Sprengel's deformity. Kidney anomalies, also mesodermal in origin, are less frequent concomitants of Sprengel's deformity. The omovertebral bone, connecting the superomedial scapula to the posterior elements of the cervical vertebrae, is found about one-quarter of the time. Various other types of disruptions of limb bud formation and differentiation can be noted, including hypoplastic thumb, clavicular hypoplasia, and hemimelias. Shoulder instability may factitiously increase shoulder motion.

Congenital elevation of the scapula is caused by an interruption in the normal caudad migration of the scapula. This produces both cosmetic and functional impairment and probably occurs between the 9th and 12th week of gestation. An arrest in the development of bone, cartilage, and muscle also occurs. The trapezius, rhomboid, or levator scapulae muscle may be absent, hypoplastic, or contain multiple fibrous adhesions. The serratus anterior muscle may be weak, leading to winging of the scapula. Other muscles, such as the pectoralis major, latissimus dorsi, or the sternocleidomastoid, may be hypoplastic and similarly involved.

Associated malformations are almost always present with a Sprengel deformity. These can include anomalies in the cervicothoracic vertebrae or the thoracic rib cage. The most common anomalies are absent or fused ribs, chest-wall asymmetry, Klippel-Feil syndrome, cervical ribs, congenital scoliosis, and cervical spina bifida. When scoliosis is present, the most common curves are in the cervicothoracic or upper thoracic region. A relationship between a Sprengel deformity and diastematomyelia has also been shown.

Another anomaly that is seen in approximately one third of patients with a Sprengel deformity is the omovertebral bone. This is a rhomboid- or trapezoid-shaped structure of cartilage or bone that usually lies in a strong fascial sheath, which extends from the superomedial border of the scapula to the spinous processes, lamina, or transverse processes of the cervical spine, most commonly the fourth to seventh cervical vertebrae. A well-developed joint can form between the scapula and the omovertebral bone; this bone can also be a solid osseous bridge. The omovertebral bone is best visualized on a lateral or oblique radiograph of the cervical spine.

Problems that may be associated with this condition include syndromes such as the following:

    Klippel-Feil syndrome.

  • Greig syndrome.

  • Poland syndrome.

  • VATER association (ie, vertebral defects, imperforate anus, tracheoesophageal fistula, and radial and renal dysplasia).

  • Velocardiofacial syndrome.

  • Floating-harbor syndrome.

  • Goldenhar syndrome.

  • X-linked dominant hydrocephalus, skeletal anomalies, and mental disturbance syndrome.

Sprengel deformity is the most common congenital malformation of the shoulder girdle. The male-to-female ratio is 3:1.

Symptoms:

Clinically, Sprengel's deformity can range from minimal, with no restriction of shoulder motion; to severe, with the superior angle of the scapula near the occiput, and marked restriction of scapulothoracic motion. Glenoid version is generally normal. The omovertebral bone, not surprisingly, is associated with greater restriction of motion. The musculature of the shoulder girdle, especially the trapezius, may be hypoplastic and/ or fibrosed.

Diagnosis:

Diagnosis may include:

    Radiographs: The Sprengel deformity is best visualized on an anteroposterior (AP) view of the chest and both shoulders.A lateral view of the cervical and thoracic spine must also be obtained to rule out associated spinal anomalies.

  • CT Scans: CT scans with 3-dimensional (3-D) reconstruction may be performed to visualize the pathoanatomy of the affected region and to visualize the omovertebral bar. CT scans may also help in planning surgery (eg, if the CT scan shows that the height-to-width ratio is markedly decreased, then the prominent convexity of the vertebral border along with the supraspinous portion of the scapula should be resected).

Treatment:

Nonoperative treatment for the Sprengel deformity consists of physical therapy. Exercises are used to maintain an individual's range of motion and to strengthen the weak periscapular muscles.

Operation is reserved for patients in whom the deformity is marked, or restriction of motion is severe; the two generally go together. Many surgical procedures have been described, including excision of the omovertebral bone, resection of the supraspinous portion of the scapula, subperiosteal resection or release of the scapula, and distal transposition of the scapula by release of the superior, osteotomy of the scapula, and medial and inferior musculature from the scapula (Green) or the their origin (Woodward), with reattachment to the scapula in its corrected position. Green originally used skeletal traction to maintain the scapula in its corrected position. Modified forms of the Green and Woodward procedure are most popular at present. Complications of surgical treatment of Sprengel's deformity include loss of correction, winging of the scapula, regeneration of the excised portion of the scapula, prominent scars, and neurovascular complications resulting from compression between the clavicle and first rib when the scapula was displaced inferiorly. Strategies to reduce complications include use of a midline incision; osteotomy or morcellization of the clavicle to increase its mobility, osteotomy of the coracoid process to release the tether of the pectoralis minor, extraperiosteal dissection to reduce bony reformation, and fasciodesis of the inferior scapula to the underlying rib to prevent winging. Results are generally satisfactory is these technical factors are integrated into the surgical plan. Lesser degrees of deformity can probably be more safely treated without morcellization or osteotomy of the clavicle.

NOTE: The above information is for processing purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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